Spontaneous regression of central nervous system posttransplant lymphoproliferative disease

摘要

Posttransplant lymphoproliferative disease(PTLD) isa group oflymphoproliferative diseases in therecipient ofsolid organ or stemcellallograft. Median timefromtransplant to PTLDisaround 4.5 years. [1] Thiscondition is highly associated withEpstein-Barr virus (EBV) infection. [2] EBV infection is present in 60%to 70%ofcases. [3] Primary central nervous system(CNS) PTLDisan extremely rareentity. Theannualincidence of PTLDis 1%to 10%and primaryCNS PTLDonly account for 5%to 15%ofallPTLDcases. [4] Pathologically, most CNS PTLDsare monomorphic, indicating poor prognosis. [1] Treatmentsuch as reduction ofimmunosuppression (RI), rituximab,chemotherapy, surgery, radiation, immunotherapy,and antiviraltherapymay showsomeeffects, [5] but long-termprognosis is far fromoptimistic. Wereportacase ofCNS polymorphic PTLD(P-PTLD) whichwas underdiagnosed 4 yearsago achieved spontaneous remission then. After being stablefor 4 years, new lesion appeared in a different brain region. Brain biopsy stillsuggested P-PTLD. Thelesion resolved after 4 rounds ofrituximab treatment.